Key Points
- Patients with VHD may present with a heart murmur, symptoms, or incidental findings of valvular abnormalities on noninvasive testing.
- The initial evaluation should include a detailed history and physical exam, ECG, chest x-ray, and transthoracic echocardiogram.
- Decisions about treatment are based primarily on the presence or absence of symptoms, severity of VHD, and response of the ventricle to pressure or volume overload imposed by VHD.
- VHD requires a multidisciplinary team and approach for its diagnosis and management.
- Reasons for valve intervention are to improve symptoms, prolong survival, and reduce the risk of complications.
- An evaluation of the surgical or interventional risk for each individual patient should be performed if intervention is indicated which includes a standard surgical risk score, along with consideration of comorbidites, frailty, and procedure specfic impediments.
- Follow-up of patients with VHD is important to assess symptom status, provide patient education, and monitor disease severity, typically with periodic echocardiography.
Table 1. The Heart Valve Team
| Recommendations | COR | LOE |
|---|---|---|
| Patients with severe VHD should be evaluated by a multidisciplinary Heart Valve Team when intervention is considered. | I | C |
Consultation with or referral to a Heart Valve Center of Excellence is reasonable when discussing treatment options for
| IIa | C |
Evaluation
Table 2. Stages of Progression of VHD
| Stage | Definition | Description |
|---|---|---|
| A | At risk | Patients with risk factors for development of VHD |
| B | Progressive | Patients with progressive VHD (mild-to-moderate severity and asymptomatic) |
| C | Asymptomatic severe | Asymptomatic patients who meet the criteria for severe VHD: C1: Asymptomatic patients with severe VHD in whom the left or right ventricle remains compensated C2: Asymptomatic patients with severe VHD, with decompensation of the left or right ventricle |
| D | Symptomatic severe | Patients who have developed symptoms as a result of VHD |
Table 3. Risk Assessment Combining STS Risk Estimate, Frailty, Major Organ System Dysfunction, and Procedure-Specific Impediments
| Type | Low Risk (Must Meet ALL Criteria in This Column) | Intermediate Risk (Any 1 Criterion in This Column) | High Risk (Any 1 Criterion in This Column) | Prohibitive Risk (Any 1 Criterion in This Column) |
|---|---|---|---|---|
| STS PROMa | <4% AND | 4%-8% OR | >8% OR | Predicted risk with surgery of death or major morbidity (all-cause) OR |
| Frailtyb | None AND | 1 index (mild) OR | â¥2 indices (moderate to severe) OR | >50% at 1 y OR |
| Major organ system compromise not to be improved postoperativelyc | None AND | 1 organ system OR | No more than 2 organ systems OR | â¥3 organ systems OR |
| Procedure-specific impedimentd | None | Possible procedure-specific impediment | Possible procedure-specific impediment | Severe procedure-specific impediment |
a Use of the STS PROM to predict risk in a given institution with reasonable reliability is appropriate only if institutional outcomes are within 1 standard deviation of STS average observed/expected ratio for the procedure in question.
b Seven frailty indices: Katz Activities of Daily Living (independence in feeding, bathing, dressing, transferring, toileting, and urinary continence) and independence in ambulation (no walking aid or assist required or 5-meter walk in <6 s). Other scoring systems can be applied to calculate no, mild-, or moderate-to-severe frailty.
c Examples of major organ system compromise: Cardiacâsevere LV systolic or diastolic dysfunction or RV dysfunction, fixed PHTN; CKD stage 3 or worse; pulmonary dysfunction with FEV1 <50% or DLCO2 <50% of predicted; CNS dysfunction (dementia, Alzheimerâs disease, Parkinsonâs disease, stroke with persistent physical limitation); GI dysfunctionâCrohnâs disease, ulcerative colitis, nutritional impairment, or serum albumin <3.0; cancerâactive malignancy; and liverâany history of cirrhosis, variceal bleeding, or elevated INR in the absence of VKA therapy.
d Examples: tracheostomy present, heavily calcified ascending aorta, chest malformation, arterial coronary graft adherent to posterior chest wall, or radiation damage.
Table 4. Initial Diagnostic Testing
| Recommendations | COR | LOE |
|---|---|---|
| TTE is recommended in the initial evaluation of patients with known or suspected VHD to confirm the diagnosis, establish etiology, determine severity, assess hemodynamic consequences, determine prognosis, and evaluate for timing of intervention. | I | B |
| TTE is recommended in patients with known VHD with any change in symptoms or physical examination findings. | I | C |
| Periodic monitoring with TTE is recommended in asymptomatic patients with known VHD at intervals depending on valve lesion, severity, ventricular size, and ventricular function. | I | C |
| Cardiac catheterization for hemodynamic assessment is recommended in symptomatic patients when noninvasive tests are inconclusive or when there is a discrepancy between the findings on noninvasive testing and physical examination regarding severity of the valve lesion. | I | C |
Exercise testing is reasonable in selected patients with asymptomatic severe VHD to
| IIa | B |
Table 5. Frequency of Echocardiograms in Asymptomatic Patients With VHD and Normal LV Function
| Valve Lesion | ||||
|---|---|---|---|---|
| Stage | ASa | AR | MS | MR |
| Progressive (stage B) | Every 3-5 y (mild severity: Vmax 2.0-2.9 m/s) Every 1-2 y (moderate severity: Vmax 3.0-3.9 m/s) | Every 3-5 y (mild severity) Every 1-2 y (moderate severity) | Every 3-5 y (MVA >1.5 cm2) | Every 3-5 y (mild severity) Every 1-2 y (moderate severity) |
| Severe (stage C) | Every 6-12 mo (Vmax â¥4 m/s) | Every 6-12 mo Dilating LV: more frequently | Every 1-2 y (MVA 1.0-1.5 cm2) Once every year (MVA <1.0 cm2) | Every 6-12 mo Dilating LV: more frequently |
Patients with mixed valve disease may require serial evaluations at intervals earlier than recommended for single valve lesions.
a With normal stroke volume.
Rheumatic Fever
Table 6. Secondary Prevention of Rheumatic Fever
| Recommendations | COR | LOE |
|---|---|---|
| Secondary prevention of rheumatic fever is indicated in patients with rheumatic heart disease, specifically MS (Tables 7-8). | I | C |
Table 7. Agents for Secondary Prevention of Rheumatic Fever
| Agent | Dosage |
|---|---|
| Penicillin G benzathine | 1.2 million units IM every 4 wka |
| Penicillin V potassium | 250 mg orally BID |
| Sulfadiazine | 1 g orally QD |
| Macrolide or azalide antibiotic (for patients allergic to penicillin and sulfadiazine)b | Varies |
| a Administration every 3 wk is recommended in certain high-risk situations. b Macrolide antibiotics should not be used in persons taking other medications that inhibit cytochrome P450 3A, such as azole antifungal agents, HIV protease inhibitors, and some selective serotonin reuptake inhibitors. Adapted with permission from Gerber et al. Circulation. 2009;119:1541-1551. | |
Table 8. Duration of Secondary Prophylaxis for Rheumatic Fever
| Type | Duration After Last Attack |
|---|---|
| Rheumatic fever with carditis and residual heart disease (persistent VHDa) | 10 y or until patient is 40 y of age (whichever is longer) |
| Rheumatic fever with carditis but no residual heart disease (no valvular diseasea) | 10 y or until patient is 21 y of age (whichever is longer) |
| Rheumatic fever without carditis | 5 y or until patient is 21 y of age (whichever is longer) |
| a Clinical or echocardiographic evidence. Adapted with permission from Gerber et al. Circulation. 2009;119:1541-1551. | |
Aortic Stenosis
Table 9. Stages of Valvular AS
| Stage | Definition | Valve Anatomy | Valve Hemodynamics | Hemodynamic Consequences | Symptoms |
|---|---|---|---|---|---|
| A | At risk of AS |
| Aortic Vmax <2 m/s | None | None |
| B | Progressive AS |
|
|
| None |
| C: Asymptomatic severe AS | |||||
| C1 | Asymptomatic severe AS | Severe leaflet calcification or congenital stenosis with severely reduced leaflet opening |
|
| None: Exercise testing is reasonable to confirm symptom status |
| C2 | Asymptomatic severe AS with LV dysfunction | Severe leaflet calcification or congenital stenosis with severely reduced leaflet opening |
| LVEF <50% | None |
| D: Symptomatic severe AS | |||||
| D1 | Symptomatic severe high- gradient AS | Severe leaflet calcification or congenital stenosis with severely reduced leaflet opening |
|
|
|
| D2 | Symptomatic severe low-flow/low-gradient AS with reduced LVEF | Severe leaflet calcification with severely reduced leaflet motion |
|
|
|
| D3 | Symptomatic severe low-gradient AS with normal LVEF or paradoxical low-flow severe AS | Severe leaflet calcification with severely reduced leaflet motion |
|
|
|
Table 10. Evaluation and Treatment in Patients With AS
| Recommendations | COR | LOE |
|---|---|---|
| Diagnostic Testing | ||
| TTE is indicated in patients with signs or symptoms of AS or a bicuspid aortic valve for accurate diagnosis of the cause of AS, hemodynamic severity, LV size and systolic function, and for determining prognosis and timing of valve intervention. | I | B |
Low-dose dobutamine stress testing using echocardiographic or invasive hemodynamic measurements is reasonable in patients with stage D2 AS with all of the following:
| I | B |
| Exercise testing is reasonable to assess physiological changes with exercise and to confirm the absence of symptoms in asymptomatic patients with a calcified aortic valve and aortic velocity â¥4.0 m/s or âPmean â¥40 mm Hg (stage C). | IIa | B |
| Exercise testing should NOT be performed in symptomatic patients with AS when the aortic velocity is â¥4.0 m/s or âPmean is â¥40 mm Hg (stage D). | III: Harm | B |
| Medical Therapy | ||
| Hypertension in patients at risk for developing AS (stage A) and in patients with asymptomatic AS (stages B and C) should be treated according to standard GDMT, started at a low dose and gradually titrated upward as needed with frequent clinical monitoring. | I | B |
| Vasodilator therapy may be reasonable if used with invasive hemodynamic monitoring in the acute management of patients with severe decompensated AS (stage D) with NYHA class IV HF symptoms. | IIb | C |
| Statin therapy is NOT indicated for prevention of hemodynamic progression of AS in patients with mild-to-moderate calcific valve disease (stages B-D). | III: No Benefit | A |
Table 11. Indications for AVR in Patients With AS
| Recommendations | COR | LOE |
|---|---|---|
AVR is recommended in symptomatic patients with severe AS (stage D1) with:
| I | B |
| AVR is recommended for asymptomatic patients with severe AS (stage C2) and LVEF <50% with decreased systolic opening of a calcified aortic valve with an aortic velocity â¥4.0 m/s or âPmean â¥40 mm Hg. | I | B |
| AVR is indicated for patients with very severe AS (stage C or D) when undergoing cardiac surgery for other indications when there is decreased systolic opening of a calcified aortic valve and aortic velocity â¥4.0 m/s or âPmean â¥40 mm Hg. | I | B |
AVR is reasonable for asymptomatic patients with severe AS (stage C1) with:
| IIa | B |
AVR is reasonable in apparently asymptomatic patients with severe AS (stage C1) with:
| IIa | B |
AVR is reasonable in symptomatic patients with low-flow/low-gradient severe AS with reduced LVEF (stage D2) with a:
| IIa | B |
AVR is reasonable in symptomatic patients with low-flow/low-gradient severe AS (stage D3) with LVEF â¥50%, a calcified aortic valve with significantly reduced leaflet motion, and a valve area â¤1.0 cm2 only if clinical, hemodynamic, and anatomic data support valve obstruction as the most likely cause of symptoms and data recorded when the patient is normotensive (systolic BP <140 mm Hg) indicate:
| IIa | C |
| AVR is reasonable for patients with moderate AS (stage B) with an aortic velocity 3.0-3.9 m/s or âPmean 20-39 mm Hg who are undergoing cardiac surgery for other indications. | IIa | C |
| AVR may be considered for asymptomatic patients with severe AS (stage C1) with aortic velocity â¥4.0 m/s or âPmean â¥40 mm Hg if the patient is at low surgical risk and serial testing shows an increase in aortic velocity â¥0.3 m/s/y. | IIb | C |
Table 12. Choice of Intervention in Patients With AS
| Recommendations | COR | LOE |
|---|---|---|
| Surgical AVR is recommended in patients who meet an indication for AVR with low or intermediate surgical risk. | I | A |
| For patients in whom TAVR or high-risk surgical AVR is being considered, a Heart Valve Team consisting of an integrated, multidisciplinary group of healthcare professionals with expertise in VHD, cardiac imaging, interventional cardiology, cardiac anesthesia, and cardiac surgery should collaborate to provide optimal patient care. | I | C |
| TAVR is recommended in patients who meet an indication for AVR who have a prohibitive risk for surgical AVR and a predicted post-TAVR survival >12 months. | I | B |
| TAVR is a reasonable alternative to surgical AVR in patients who meet an indication for AVR and who have high surgical risk for surgical AVR. | IIa | B |
| Percutaneous aortic balloon dilation may be considered as a bridge to surgical AVR or TAVR in patients with severe symptomatic AS. | IIb | C |
| TAVR is NOT recommended in patients in whom existing comorbidities would preclude the expected benefit from correction of AS. | III: No Benefit | B |
Figure 1. Indications for AVR in Patients With AS
Table 13. Stages of Chronic AR
| Stage | Definition | Valve Anatomy | Valve Hemodynamics | Hemodynamic Consequences | Symptoms |
|---|---|---|---|---|---|
| A | At risk of AR |
| AR severity: none or trace | None | None |
| B | Progressive AR |
| Mild AR:
Moderate AR:
|
| None |
| C | Asymptomatic severe AR |
| Severe AR:
| C1:
C2:
| None; exercise testing is reasonable to confirm symptom status |
| D | Symptomatic severe AR |
| Severe AR:
|
|
|
Table 14. Diagnosis, Medical Therapy, and Intervention in Patients With Chronic AR
| Recommendations | COR | LOE |
|---|---|---|
| TTE is indicated in patients with signs or symptoms of AR (stages A-D) for accurate diagnosis of the cause of regurgitation, regurgitant severity, and LV size and systolic function, and for determining clinical outcome and timing of valve intervention. | I | B |
| TTE is indicated in patients with dilated aortic sinuses or ascending aorta or with a bicuspid aortic valve (stages A and B) to evaluate the presence and severity of AR. | I | B |
| CMR is indicated in patients with moderate or severe AR (stages B-D) and suboptimal echocardiographic images for the assessment of LV systolic function, systolic and diastolic volumes, and measurement of AR severity. | I | B |
| Medical Therapy | ||
| Treatment of hypertension (systolic BP >140 mm Hg) is recommended in patients with chronic AR (stages B and C), preferably with dihydropyridine calcium channel blockers or ACE inhibitors/ARBs. | I | B |
| Medical therapy with ACE inhibitors/ARBs and beta blockers is reasonable in patients with severe AR who have symptoms and/or LV dysfunction (stages C2 and D) when surgery is not performed because of comorbidities. | IIa | B |
| Surgical Intervention | ||
| AVR is indicated for symptomatic patients with severe AR regardless of LV systolic function (stage D). | I | B |
| AVR is indicated for asymptomatic patients with chronic severe AR and LV systolic dysfunction (LVEF <50%) at rest (stage C2) if no other cause for systolic dysfunction is identified. | I | B |
| AVR is indicated for patients with severe AR (stage C or D) while undergoing cardiac surgery for other indications. | I | C |
| AVR is reasonable for asymptomatic patients with severe AR with normal LV systolic function (LVEF â¥50%) but with severe LV dilation (LVESD >50 mm or indexed LVESD >25 mm/m2) (stage C2). | IIa | B |
| AVR is reasonable in patients with moderate AR (stage B) while undergoing surgery on the ascending aorta, CABG, or MV surgery. | IIa | C |
| AVR may be considered for asymptomatic patients with severe AR and normal LV systolic function at rest (LVEF â¥50%, stage C1) but with progressive severe LV dilation (LVEDD >65 mm) if surgical risk is low. | IIb | C |
